Arterial aneurysm and dissection: toward the evolving phenotype of Tatton-Brown-Rahman syndrome

Tatton-Brown Rahman syndrome (TBRS) is a recently described rare disease. The knowledge contributed by new patients is crucial for enhancing our understanding of the condition and, consequently, improving its management and follow-up.  The prevalence of aortic disease in TBRS individuals is far in excess of that expected in the general population. This cohort, together with individuals previously published, illustrates the importance to consider dilatation/dissection, mainly in aorta but also in other arteries. Our study emphasizes the importance of indicating ongoing cardiovascular monitoring in patients with TBRS, as it represents a manifestation with high morbidity and mortality. It also underscores the relevance of patient follow-up at different life stages. Understanding the evolving clinical manifestations contributes to enhancing overall patient care. (By Dr. Irene Valenzuela Palafoll, https://jmg.bmj.com/content/early/2024/07/02/jmg-2024-109861 )

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